ArticleMolecular biology of the cell2015
The rod domain is not essential for the function of plectin in maintaining tissue integrity.
Article in Molecular biology of the cell, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 22 citations in OpenAlex.
- Mechanics of cell sheets: plectin as an integrator of cytoskeletal networks.Open biology · 2025Review
- Cancer cell extravasation requiresBritish journal of cancer · 2024Observational
- Homozygosity for a RareInternational journal of molecular sciences · 2024Article
- Dystonin modifiers of junctional epidermolysis bullosa and models of epidermolysis bullosa simplex without dystonia musculorum.PloS one · 2023Article
- Junctional epithelium and hemidesmosomes: Tape and rivets for solving the "percutaneous device dilemma" in dental and other permanent implants.Bioactive materials · 2022Review
- Mutation update: The spectra of PLEC sequence variants and related plectinopathies.Human mutation · 2022Article
- Expression of risk genes linked to vitamin D receptor super-enhancer regions and their association with phenotype severity in multiple sclerosis.Frontiers in neurology · 2022Article
- Therapeutic Prospects of Exon Skipping for Epidermolysis Bullosa.International journal of molecular sciences · 2021Review
- Plectin in Skin Fragility Disorders.Cells · 2021Review
- Muscle-Related Plectinopathies.Cells · 2021Review
- Review
- An inside look at a biofilm:Science advances · 2021Article
- A prometastatic splicing program regulated by SNRPA1 interactions with structured RNA elements.Science (New York, N.Y.) · 2021Article
- Integrin α3β1 in hair bulge stem cells modulates CCN2 expression and promotes skin tumorigenesis.Life science alliance · 2020Article
- The Structure of the Plakin Domain of Plectin Reveals an Extended Rod-like Shape.The Journal of biological chemistry · 2016Article
- Downstream effects of plectin mutations in epidermolysis bullosa simplex with muscular dystrophy.Acta neuropathologica communications · 2016Article
- Co-Orientation: Quantifying Simultaneous Co-Localization and Orientational Alignment of Filaments in Light Microscopy.PloS one · 2015Article
Corrections and comments
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Authors and funding
8 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Epidermolysis bullosa simplex associated with late-onset muscular dystrophy (EBS-MD) is an autosomal recessive disorder resulting from mutations in the plectin gene. The majority of these mutations occur within the large exon 31 encoding the central rod domain and leave the production of a low-level rodless plectin splice variant unaffected. To investigate the function of the rod domain, we generated rodless plectin mice through conditional deletion of exon 31. Rodless plectin mice develop normally without signs of skin blistering or muscular dystrophy. Plectin localization and hemidesmosome organization are unaffected in rodless plectin mice. However, superresolution microscopy revealed a closer juxtaposition of the C-terminus of plectin to the integrin β4 subunit in rodless plectin keratinocytes. Wound healing occurred slightly faster in rodless plectin mice than in wild-type mice, and keratinocytes migration was increased in the absence of the rod domain. The faster migration of rodless plectin keratinocytes is not due to altered biochemical properties because, like full-length plectin, rodless plectin is a dimeric protein. Our data demonstrate that rodless plectin can functionally compensate for the loss of full-length plectin in mice. Thus the low expression level of plectin rather than the absence of the rod domain dictates the development of EBS-MD.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.